Lutembacher Syndrome Treatment & Management

Updated: Mar 26, 2014
  • Author: Kamran Riaz, MD; Chief Editor: Park W Willis IV, MD  more...
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Treatment

Medical Care

See the list below:

  • Symptomatic relief
    • Right-sided heart failure - Diuretics
    • Atrial arrhythmias - Digoxin, beta-blockers, and calcium channel blockers used mainly for rate control, while amiodarone and sotalol used not only for rate control but also for conversion into and maintenance of normal sinus rhythm
  • Subacute bacterial endocarditis (SBE) prophylaxis: Patients with Lutembacher syndrome who have undergone complete repair with prosthetic material or a device need SBE prophylaxis for the first 6 months after the repair procedure. In addition, patients with previous history of endocarditis warrant SBE prophylaxis.
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Surgical Care

Until recently, surgery was the only definite curative treatment available and involved closure of the ASD and mitral commissurotomy or mitral valve replacement. [5]

  • Percutaneous closure of ASD and mitral balloon valvuloplasty [6, 7, 8, 9, 10]
    • Percutaneous closure of the ASD with a clamshell device and mitral valvuloplasty provides a nonsurgical approach to correct these defects. Although mitral valvuloplasty has been performed for several decades, percutaneous closure of an ASD with a device represents a still-developing technology.
    • As already described, mitral valvuloplasty alone can be complicated by development of ASD secondary to transseptal puncture performed as a part of the procedure.
  • Indications for surgery or percutaneous intervention
    • ASD with a Qp/Qs ratio of more than 1.5
    • Moderate-to-severe mitral stenosis
    • Any degree of pulmonary hypertension, except individuals with irreversible pulmonary hypertension (Eisenmenger syndrome, see below)
  • Surgery is now performed early rather than late because the rates of heart failure and cardiac arrhythmia increase with age. Patients with pulmonary hypertension should demonstrate reversibility of pulmonary vascular resistance prior to surgical (or percutaneous) correction of ASD. Patients with pulmonary hypertension and irreversibly increased pulmonary vascular resistance (ie, Eisenmenger physiology) invariably develop progressive right-sided heart failure after ASD closure and die.
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Consultations

See the list below:

  • Cardiothoracic surgeon
  • Interventional cardiologist
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Diet

Patients should adhere to a low-sodium diet.

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Activity

Activity should be as tolerated by the patient.

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